Extended Sick Neonate Score (ESNS) for Clinical Assessment and Mortality Prediction in Sick Newborns referred to Tertiary Care

Objective:To evaluate utility of a new Extended Sick Neonate Score (ESNS). to predict ‘in-hospital mortality’ and compare with Score for Neonatal Acute Physiology – PerinatalExtension II (SNAPPE II) and Sick Neonate Score (SNS). Design:Prospective observationalstudy. Methods:All extramural sick newborns transported to the neonatology unit of a tertiarycare teaching hospitalover a period of one year.Correlation between ESNS, SNAPPE-II andSNS scoring, and sensitivity/specificity of each score to predict mortality were determined.Results:961 newborns were enrolled in the study. ESNS, SNAPPE II and SNS were stronglycorrelated, even when stratified by gestation. ESNS of ≤11 had the best sensitivity (85.9%)and specificity (89.8%). For preterms, ESNS ≤12 had the best sensitivity (92.3%) andspecificity (76.7%). Conclusion:ESNS can predict ‘in-hospital mortality’ outcome withsatisfactory sensitivity and specificity

Clitoral Length and Anogenital Ratio in Indian Newborn Girls.

Objective: To generate normative data on clitoris length, anogenital distance and anogenital ratio in Indian newborns. Design: Cross-sectional study. Setting: Neonatal unit of a tertiary care teaching hospital in Kolkata. Participants: 378 female neonates, who were hemo-dynamically stable without critical illness or chromosomal anomaly, and without any vulval hematoma or genital abnormalities. Interventions: Measurements were recorded using a digital vernier caliper between 24-72 hours. Infant was held in position by an assistant, while the investigator measured clitoral length by gently retracting the labia majora. Anogenital distance (centre of the anus to posterior convergence of the fourchette) and anogenital ratio (anogenital distance divided by the distance from centre of the anus to base of the clitoris) was also measured. Main outcome measures: Gestational age- and birthweight-wise normative values of clitoral length, anogenital distance and anogenital ratios. Results: Mean clitoral length was 3.1 (1.54) mm for the whole cohort while anogenital distance and anogenital ratio were 10.2 (2.78) mm and 0.34 (0.07) mm, respectively. The gestation age-wise percentile charts of clitoral length, anogenital distance and anogenital ratio have been generated. There was no correlation between clitoral length and gestational age, body length, head circumference and birth weight. Correlations were also weak for anogenital distance. Conclusions: The normative values generated can serve as reference standard in the assessment of clitoromegaly, ambiguous genitalia, virilizing effects and suspected in utero androgen exposure.

Normative Blood Pressure Data for Indian Neonates.

Objective: To establish the normative blood pressure (BP) values in healthy Indian neonates using oscillometric method, and to develop BP percentile charts. Design: Prospective observational study. Setting: Neonatal unit of a teaching hospital in Eastern India. Participants: 1617 hemodynamically stable inborn neonates without birth asphyxia, major congenital anomaly, maternal complications (e.g. preeclampsia, hypertension, diabetes) or critical neonatal illness. Procedure: Quite state measurements of systolic BP (SBP), diastolic BP (DBP) and mean arterial pressure (MAP) were recorded by oscillometric method on day 4, 7 and 14 of postnatal life. The averages of three readings at 2-minute intervals were used. Results: Percentile charts (providing 5th, 10th, 25th, 50th, 75th, 95th, and 99th percentile values) have been developed. SBP, DBP and MAP showed a steady rise from day 4 to day 14, and were comparable between males and females, but were significantly lower in preterms than in term neonates Conclusions: Normative neonatal BP data along with gestational age-wise percentile charts shall be of help for decision-making and planning for sick newborns.

Dorfman-Chanarin syndrome: A rare neutral lipid storage disease.

Dorfman-Chanarin syndrome is a rare neutral lipid storage disorder characterized by ichthyosis, lipid vacuolations in peripheral leucocytes, and multisystem involvement. It is an autosomal recessive disorder caused by mutations in the CGI-58 gene. A total of 42 cases have been reported worldwide till February 2009 out of which 4 have been previously reported from India. We report a case of a 20-month-old male with congenital ichthyosis, organomegaly, and bilateral cryptorchidism. Examination of the peripheral smear revealed lipid vacuoles in the leucocytes consistent with Jordan's anomaly, which was confirmed by transmission electron microscopy. Liver biopsy revealed micronodular cirrhosis with macrovesicular steatosis while skin biopsy showed ichthyosis vulgaris. Dorfman-Chanarin syndrome was diagnosed on the basis of clinical and laboratory criteria with certain unreported manifestations. Dietary modifications were instituted and followed up after 1 year with promising results. This emphasizes the importance of neonatal screening for lipid vacuolations in peripheral blood in all cases of congenital ichthyosis.